Potassium voltage-gated channel subfamily KQT member 4

UniProt P56696 Organism Homo sapiens Gene KCNQ4

Also known as: KCNQ4

Function

Pore-forming subunit of the voltage-gated potassium (Kv) channel involved in the regulation of sensory cells excitability in the cochlea. KCNQ4/Kv7.4 channel is composed of 4 pore-forming subunits assembled as tetramers. Promotes the outflow of potassium ions in the repolarization phase of action potential which plays a role in regulating membrane potential of excitable cells. The channel conducts a slowly activating and deactivating current. Current often shows some inward rectification at positive potentials. Channel may be selectively permeable in vitro to other cations besides potassium, in decreasing order of affinity K(+) = Rb(+) > Cs(+) > Na(+). Important for normal physiological function of inner ear such as sensory perception of sound.

Classification

Family (Pfam): PF00520 Ion_trans, PF03520 KCNQ_channel
InterPro: Ion_trans_dom, K_chnl_volt-dep_KCNQ, K_chnl_volt-dep_KCNQ_C
Functional cluster: Ribosome-Biogenesis GTPases (Obg/Era/Der)

Experimental structures · PDB · 13

2OVC X-ray 2.07A
4GOW X-ray 2.60A
6B8L X-ray 2.30A
6B8M X-ray 2.30A
6B8N X-ray 2.20A
6B8P X-ray 2.20A
6N5W X-ray 2.15A
7BYL EM 2.50A
7BYM EM 3.10A
7BYN EM 3.30A
7VNP EM 2.79A
7VNQ EM 2.96A
… and 1 more

A predicted model is available from AlphaFold.

Gene Ontology · 8

Disease associations

autosomal dominant nonsyndromic hearing loss 2A MONDO:0010817

Drugs targeting this protein · 8

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

Mu-scoloptoxin(15)-Ssm1a 1 shared papers
Potassium voltage-gated channel subfamily KQT member 5 1 shared papers
Heat shock protein HSP 90-beta 1 shared papers
Potassium voltage-gated channel subfamily KQT member 3 1 shared papers
Potassium voltage-gated channel subfamily KQT member 2 1 shared papers
Potassium voltage-gated channel subfamily KQT member 1 1 shared papers

Literature · 13 cited papers

Structural insights into the lipid and ligand regulation of a human neuronal KCNQ channel. Neuron · 2022
A calmodulin C-lobe Ca(2+)-dependent switch governs Kv7 channel function. Neuron · 2018
Centipedes subdue giant prey by blocking KCNQ channels. Proc. Natl. Acad. Sci. U.S.A. · 2018
Distinct roles of molecular chaperones HSP90alpha and HSP90beta in the biogenesis of KCNQ4 channels. PLoS ONE · 2013
Autosomal dominant progressive sensorineural hearing loss due to a novel mutation in the KCNQ4 gene. Arch. Otolaryngol. Head Neck Surg. · 2011
Structural insight into KCNQ (Kv7) channel assembly and channelopathy. Neuron · 2007
The DNA sequence and biological annotation of human chromosome 1. Nature · 2006
KCNQ4 channels expressed in mammalian cells: functional characteristics and pharmacology. Am. J. Physiol. · 2001
Mutations in the KCNQ4 K+ channel gene, responsible for autosomal dominant hearing loss, cluster in the channel pore region. Am. J. Med. Genet. · 2000
Inhibition of KCNQ1-4 potassium channels expressed in mammalian cells via M1 muscarinic acetylcholine receptors. J. Physiol. (Lond.) · 2000
Novel mutation in the KCNQ4 gene in a large kindred with dominant progressive hearing loss. Hum. Mutat. · 1999
Mutations in the KCNQ4 gene are responsible for autosomal dominant deafness in four DFNA2 families. Hum. Mol. Genet. · 1999
… and 1 more in the literature graph

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